From the Guidelines
For patients with von Willebrand disease undergoing surgery, desmopressin (DDAVP) at a dose of 0.3 μg.kg-1 may be considered to stimulate release of von Willebrand factor (vWF) from the endothelium, especially in those with acquired or inherited vWF deficiency. This approach is based on the most recent guidelines from the Association of Anaesthetists, which suggest the use of desmopressin as a pro-haemostatic drug for patients with inherited bleeding disorders 1.
Key Considerations
- Desmopressin has been evaluated in the peri-operative setting and may lead to a small reduction in blood loss and volume of RBCs transfused in patients undergoing cardiac surgery, although the clinical importance of these differences is uncertain 1.
- Patients who may benefit from desmopressin include those with platelet dysfunction secondary to cardiopulmonary bypass or recent antiplatelet therapy prior to cardiac surgery, but the certainty of the evidence is low 1.
- For more severe cases or Type 2 and 3 VWD, von Willebrand factor (VWF) concentrate such as Humate-P or Wilate may be recommended, with treatment aiming to achieve VWF levels of at least 50% for minor procedures and 100% for major surgeries.
Management Approach
- Laboratory monitoring of VWF:RCo and Factor VIII levels is important before surgery and potentially during recovery.
- Antifibrinolytic agents like tranexamic acid may be used as adjunctive therapy.
- Patients should discontinue medications that affect platelet function (aspirin, NSAIDs) 7-10 days before surgery.
- A hematology consultation is strongly recommended for individualized treatment planning, as response to therapy varies between patients 1.
Postoperative Care
- Postoperative monitoring and potential maintenance therapy may be needed for 7-14 days depending on the surgical procedure and healing requirements.
- The use of desmopressin and other therapies should be guided by the most recent and highest quality evidence, with consideration of individual patient factors and the specific surgical procedure 1.
From the FDA Drug Label
Desmopressin acetate injection 4 mcg/mL is indicated for patients with mild to moderate classic von Willebrand’s disease (Type I) with factor VIII levels greater than 5% Desmopressin acetate injection will often maintain hemostasis in patients with mild to moderate von Willebrand’s disease during surgical procedures and postoperatively when administered 30 minutes prior to the scheduled procedure
Desmopressin acetate injection can be used before surgery in patients with mild to moderate von Willebrand’s disease (Type I) to maintain hemostasis, when administered 30 minutes prior to the scheduled procedure 2.
- Key considerations:
- Factor VIII levels: greater than 5%
- Type of von Willebrand’s disease: mild to moderate classic von Willebrand’s disease (Type I)
- Administration timing: 30 minutes prior to the scheduled procedure
- Monitoring: Bleeding time and factor VIII coagulant activity, ristocetin cofactor activity, and von Willebrand factor antigen should be checked during administration of desmopressin acetate injection to ensure that adequate levels are being achieved.
From the Research
Pre-Surgical Management of Von Willebrand Disease
- Patients with Von Willebrand disease (VWD) require careful perioperative management to minimize bleeding risk, as surgical procedures represent a serious hemostatic challenge 3.
- Risk stratification includes the nature of the surgery, baseline plasma von Willebrand factor (VWF) levels, bleeding history, and responses to previous challenges 3.
- Baseline bleeding scores (BSs) may assist in identification of patients with a higher risk of postsurgical bleeding 3.
Treatment Options
- Desmopressin (DDAVP)-stimulated release of endogenous VWD is an effective treatment strategy in many patients, but plasma concentrates containing VWF are the preferred option for most patients undergoing surgical procedures 4.
- Treatment guidelines recommend the use of pure von Willebrand factor or von Willebrand factor/factor VIII (vWF/FVIII) concentrate in patients with type 2 or type 3 VWD undergoing surgery, and in patients with type 1 VWD undergoing surgery who are unresponsive to desmopressin 5.
- VWF/FVIII concentrates should be assessed using parameters such as FVIII:coagulant activity, VWF:ristocetin cofactor activity, VWF:collagen binding, and VWF multimeric patterns to determine their predicted efficacy and safety 6.
Perioperative Care
- Hemostatic levels are maintained until bleeding risk abates, usually 3 to 5 days for minor procedures and 7 to 14 days for major surgery 3.
- Frequent monitoring of plasma levels and access to hemostatic testing are essential for patients undergoing major surgery, particularly with more severe forms of VWD 3.
- A shared-decision model leading to individualized therapy plans is important in patients with VWD who are undergoing surgical and invasive procedures, given the low-quality evidence for guiding management decisions 7.